Yonke into omele uyazi ngePorphyria Cutanea Tarda

Umxholo

• Iimpawu
• Imifanekiso ye porphyria cutanea tarda
• Oonobangela
• Imiba yomngcipheko
• Iziganeko
• Uxilongo
• Unyango
• Imbonakalo

Ushwankathelo

I-Porphyria cutanea tarda (PCT) luhlobo lwe-porphyria okanye isifo segazi esichaphazela ulusu. I-PCT yenye yeentlobo eziqhelekileyo ze-porphyria. Ngamanye amaxesha kubhekiswa kwi-colloquially njengesifo se-vampire. Kungenxa yokuba abantu abanale meko bahlala befumana iimpawu ezilandela ukukhanya kwelanga.

Iimpawu

Uninzi lweempawu ze porphyria cutanea tarda zivela kulusu. Iimpawu eziqhelekileyo zibandakanya:

• Amadyungudyungu kulusu oluvezwe lilanga, kubandakanya izandla, ubuso neengalo
• photosensitivity, oko kuthetha ukuba ulusu lwakho lubuthathaka elangeni
• ulusu olubhityileyo okanye olubuthathaka
• ukukhula kweenwele, zihlala ebusweni
• Ukuqhaqha kunye nokukhanda kwesikhumba
• ubomvu, ukudumba, okanye ukurhawuzelela ulusu
• izilonda ezikhulayo emva kokonzakala okuncinci kulusu
• i-hyperpigmentation, okuthetha ukuba amabala esikhumba aba mnyama
• umchamo omnyama kunesiqhelo okanye umbala obomvu umdaka
• ukulimala kwesibindi

Emva kokuba i-blisters ifom kulusu lwakho, ulusu lunokuchuba. Kuqhelekile nokuba kubonakale ukubonakala xa i-blisters iphilisa.

Iipatches ze-Hyperpigmentation patches zihlala zibonakala ebusweni, ezandleni nasentanyeni.

Imifanekiso ye porphyria cutanea tarda

Oonobangela

I-Porphyria cutanea tarda inokubangelwa zizinto ezahlukeneyo. Izizathu zihlala zihlulwa njengezofuzo okanye ezifunyenweyo.

Izizathu eziqhelekileyo zofuzo zibandakanya:

• Imbali yosapho ye porphyria cutanea tarda
• Ukusilela kwilifa le-enzyme yesibindi uroporphyrinogen decarboxylase
• Imbali yosapho yesifo sesibindi okanye umhlaza wesibindi
• intsimbi yesibindi ngaphezu kwesiqhelo

Ezona zizathu zixhaphakileyo ezifunyenweyo zibandakanya:

• utywala
• usebenzisa unyango lwe-estrogen
• usebenzisa izinto zokucwangcisa ngomlomo
• ukubonakaliswa kwezinto ezithile zokusingqongileyo okanye iikhemikhali, ezinje ngeAgent Orange
• ukuthatha i-iron eninzi kakhulu
• ukutshaya
• ukuba ne-hepatitis C
• une-HIV

Ngamanye amaxesha, akunakwenzeka ukumisela unobangela we-porphyria cutanea tarda.

Imiba yomngcipheko

Usemngciphekweni omkhulu we-porphyria cutanea tarda ukuba uyatshaya okanye usebenzisa utywala. Kusenokwenzeka ukuba ufumane le meko ukuba une-hepatitis C okanye i-HIV.

Ukuvezwa kwiikhemikhali ezithile, ezinje ngeAgent Orange, nako kunokunyusa umngcipheko. Usenokuba ubonakalisiwe kule khemikhali ukuba uligqala elalisebenza kwindawo eyayineAgent Orange.

Iziganeko

I-Porphyria cutanea tarda inokuchaphazela amadoda nabafazi. Ngokuqhelekileyo kubonakala emva kweminyaka engama-30, ngoko akuqhelekanga phakathi kwabantwana okanye abaselula.

I-Porphyria cutanea tarda ichaphazela abantu kwihlabathi liphela kwaye ayikhawulelwanga kwingingqi ethile okanye ilizwe. Kuqikelelwa ukuba umntu omnye kwabali-10 000 ukuya kuma-25,000 unale meko.

Uxilongo

Ugqirha wakho angenza uvavanyo lomzimba, ajonge iimpawu, kwaye arekhode imbali yakho yonyango. Ukongeza, banokusebenzisa ezi mvavanyo zilandelayo ukufumanisa i-porphyria cutanea tarda:

• iimvavanyo zegazi
• iimvavanyo zomchamo
• Uvavanyo lwelindle
• ulusu biopsy

Ugqirha uza kujonga amanqanaba akho e-porphyrin kunye nee-enzyme zesibindi. Ukuvavanywa kwemfuza kunokucetyiswa kubantu abanembali yosapho yale meko.

Unyango

Unyango lwe-porphyria cutanea tarda igxile ekulawuleni nasekumiseni iimpawu. Utshintsho kwindlela yokuphila, njengokunciphisa ukusela utywala kunye nokungatshayi, kunokunceda.

Izinketho zonyango eziqhelekileyo zibandakanya:

• phlebotomy, kukususwa kwegazi ukunciphisa i-iron
• chloroquine (Aralen)
• ihydroxychloroquine (iPlaquenil)
• amayeza entlungu
• I-iron chelators
• Ukunyanga izifo ezibangela i-porphyria cutanea tarda, efana ne-HCV okanye i-HIV

I-Phlebotomy lolunye lonyango oluqhelekileyo lwe-porphyria cutanea tarda. Iipilisi ze-Antimalarial nazo zisetyenziswa rhoqo.

Iinguqu eziqhelekileyo kwindlela yokuphila ukunyanga i-porphyria cutanea tarda zibandakanya:

• ukuphepha utywala
• engatshayi
• ukuphepha ukukhanya kwelanga
• usebenzisa i-sunscreen
• ukunqanda ukwenzakala esikhumbeni
• ungathathi ii-estrogens

Kuya kufuneka unxibe i-sunscreen, imikhono emide, kunye nomnqwazi ukunqanda ilanga.

I-Porphyria cutanea tarda inokunyusa umngcipheko womhlaza wesibindi okanye i-cirrhosis, enesiva esibindini. Yiyo loo nto kubalulekile ukuba ungaseli tywala ukuba unale meko.

Imbonakalo

I-Porphyria cutanea tarda ihlala ichaphazela abantu abadala abadala kunama-30. Sisifo segazi esichaphazela ikakhulu ulusu. Ulusu lwakho lunokuba nobuthathaka elangeni, kungoko kufuneka uthabathe amanyathelo okhuseleko ukuthintela ilanga. Amadyungudyungu aqhelekile kule meko.

Ugqirha wakho unokucebisa ngeendlela ezahlukeneyo zonyango lwe-porphyria cutanea tarda. Iipilisi ze-Phlebotomy kunye ne-antimalarial zezona zikhetho zonyango ziqhelekileyo.

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